RESUMEN
Renal lymphangiectasia is a rare cystic kidney disorder. This disorder has been termed renal lymphangioma, renal lymphangiomatosis, pararenal lymphatic cyst. We report on a 32-year-old man admitted with complaints of abdominal discomfort, in whom unilateral renal lymphangiectasia with renal vein thrombosis was diagnosed. Abdominal computed tomography revealed left perirenal fluid collection with multiloculation. Left renal vein was completely obliterated due to thrombosis and inferior vena cava (IVC) was partially obliterated. Perirenal fluid collection and renal vein thrombosis were treated initially with percutaneous drainage and anticoagulation therapy. The biochemical features of the drained fluid were compatible with lymphatic fluid. Since there was no decrease in the amount of daily drainage, left nephrectomy and IVC thrombectomy was performed on the 34th day of hospitalization. Left kidney was enlarged (15x10x18 cm, 750 g) and composed of numerous smooth, thin-walled cysts. Pathologic diagnosis was renal lymphangiectasia.